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relies on this pathway to efficiently direct synthetic enzymes to the lysosome where each can metabolize its particular substrate. The pH in the late endosome can reach 6.0, which causes dissociation of M6P from its receptor. Upon release, the enzymes are ferried to their final destination in the
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Coutinho, MF; Prata, MJ (2011-12-15). "Mannose-6-phosphate pathway: A review on its role in lysosomal function and dysfunction".
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residues with M6P. Once appropriately marked with the M6P targeting signal, these proteins are moved to the
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455:
274:
InChI=1S/C6H13O9P/c7-3-2(1-14-16(11,12)13)15-6(10)5(9)4(3)8/h2-10H,1H2,(H2,11,12,13)/t2-,3-,4+,5+,6?/m1/s1
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lysosomes. The MPRs are packed into vesicles that bud off the late endosome and return to the
424:
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Except where otherwise noted, data are given for materials in their
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The M6P-tagged lysosomal enzymes are shipped to the late
497:-Golgi network. In this way, the MPRs can be recycled.
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775:
701:
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431:. The M6P tag is added to such proteins in the
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553:(4th ed.). New York: Garland Science.
452:N-acetylglucosamine-1-phosphate transferase
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622:Role of M6P in protein modification(video)
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614:at the U.S. National Library of Medicine
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439:. Specifically, in a reaction involving
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512:Insulin-like growth factor 2 receptor
277:Key: NBSCHQHZLSJFNQ-QTVWNMPRSA-N
120:
7:
549:Alberts, Bruce; et al. (2002).
427:that are destined for transport to
187:
420:M6P is a key targeting signal for
14:
576:Molecular Genetics and Metabolism
298:C(C1C(C(C(C(O1)O)O)O)O)OP(=O)(O)O
360:
22:
469:-Golgi network. There, the M6P
356:(at 25 °C , 100 kPa).
477:(MPR) proteins at pH 6.5–6.7.
1:
740:Uridine diphosphate galactose
551:Molecular biology of the cell
612:Mannose-6-Phosphate+Receptor
475:mannose 6-phosphate receptor
745:Uridine diphosphate glucose
588:10.1016/j.ymgme.2011.12.012
473:is recognized and bound by
415:mannose phosphate isomerase
346:260.136 g/mol
831:
810:Monosaccharide derivatives
693:Glyceraldehyde 3-phosphate
490:lysosomal storage diseases
486:Enzyme replacement therapy
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735:
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484:via vesicular transport.
401:) is a molecule bound by
350:
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303:O=P(O)(O)OC1OC(O)(O)(O)1O
286:
261:
145: Unspecified anomers
53:
35:
30:
21:
616:Medical Subject Headings
582:(4). Elsevier: 542–550.
665:metabolic intermediates
409:. M6P is converted to
715:Galactose-1-phosphate
459:-linked glycosylation
789:Fructose 6-phosphate
727:Fructose 6-phosphate
681:Fructose-1-phosphate
411:fructose 6-phosphate
17:Mannose 6-phosphate
785:Mannose-6-phosphate
723:Glucose 6-phosphate
719:Glucose 1-phosphate
522:Mannose 1-phosphate
441:uridine diphosphate
395:Mannose-6-phosphate
176:mannose-6-phosphate
18:
488:(ERT) for several
448:-acetylglucosamine
425:precursor proteins
383:Infobox references
153: alpha anomer
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560:978-0-8153-3218-3
391:Chemical compound
389:
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230:CompTox Dashboard
161: beta anomer
102:Interactive image
95:Interactive image
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815:Organophosphates
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319:Chemical formula
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437:Golgi apparatus
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689:Glyceraldehyde
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606:External links
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454:catalyzes the
422:acid hydrolase
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48:-mannopyranose
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407:immune system
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54:Identifiers
41:
312:Properties
122:CHEBI:48066
44:-Phosphono-
804:Categories
758:Galactitol
528:References
463:asparagine
443:(UDP) and
342:Molar mass
218:U2S53JF879
133:ChemSpider
82:3D model (
61:CAS Number
37:IUPAC name
703:Galactose
662:galactose
482:endosomes
429:lysosomes
71:3672-15-9
673:Fructose
658:Fructose
596:22266136
501:See also
336:P
777:Mannose
517:Mannose
405:in the
376:what is
374: (
185:PubChem
763:Iditol
618:(MeSH)
594:
557:
471:moiety
403:lectin
371:verify
368:
291:SMILES
158:388338
150:394282
31:Names
495:trans
467:trans
266:InChI
198:65127
142:58636
113:ChEBI
84:JSmol
685:DHAP
660:and
592:PMID
555:ISBN
209:UNII
170:MeSH
584:doi
580:105
461:of
433:cis
413:by
399:M6P
235:EPA
188:CID
806::
787:→
725:→
721:→
717:→
691:→
683:→
590:.
578:.
535:^
417:.
330:13
40:6-
687:/
650:e
643:t
636:v
598:.
586::
563:.
457:N
446:N
435:-
397:(
366:Y
334:9
332:O
328:H
326:6
324:C
237:)
233:(
86:)
46:D
42:O
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